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Origin Story

Hello and Happy New Year! I'm Alex Lach and my fiance, Brittany, was diagnosed with GPA in October 2018. I've been so inspired reading all the posts on this forum. It feels amazing to have a community of so many people that understand what we're going through! At times it feels really hard to explain to family and friends what this diagnosis entails.

Brittany and I met in college six years ago. I found a job in the oil industry in Houston and when Brittany finished graduate school she joined me in here. Before her diagnosis she was working for a NASA contractor. Right now things are in limbo with her work. Her short term disability just ended so she's trying to determine if she'll be able to work from home part time, go on long-term disability, or go back to work full time. The biggest part of all this is figuring out how to maintain insurance coverage, since her current plan has been a literal life-saver.

Right now we're on the journey of figuring out long-term treatment (i.e. rtx or aza) and trying to understand this strange disease. I'm really looking forward to talking with people on this forum about the science of GPA as well as the latest research into treatment!

The full "origin story" of Brittany's diagnosis and treatment is below, but here's the tl;dr: Brittany coughed blood, went to hospital, got abx and left hospital, went back to hospital, had positive ANA and ANCA, got prednisone, left hospital, got 4x rituximab, and now is tapering prednisone and taking Imuran.

Brittany has had a history of asthma and allergies and got allergy shots in early/mid 2018. In September 2018 her eyes became bloodshot. After seeing an ophthalmologist she was prescribed steroid eye drops, which cleared things up for a while, though it came back by the end of the month.

In early October Brittany was traveling to Arizona for work and she coughed up a spot of blood after a workout. Two days later she was back in Houston and it happened again but with more blood. She went to a clinic. They sent her to the ER and I left work to meet her. At this point she was coughing up a scary/significant amount of blood. She was referred to St. Lukes hospital and admitted that night (October 4). Over the next six days doctors ran every test under the sun while I stayed with her in the hospital. Her ANA came back 1:2560 with a nucleolar pattern. There was trace blood and protein in her urine. Her MPO and PR3 came back normal. GBM normal. C3 and C4 normal. Sjogren's normal. She was treated with IV antibiotics and showed gradual improvement. Bronchoscopy on October 9 showed only old blood in the lungs in the areas they checked. On October 10 they released her with oral abx. At this point the cough was gone and we thought our ordeal was over...

The next day (October 11) she saw a rheumatologist, Dr. Kalpana Bhairavarasu, at BCM, who recommended repeating lab work next week and said to go to the ER if any symptoms worsened over the weekend.

On Saturday October 13, Brittany's birthday, the cough (with blood) came back. We went to the St. Lukes ER. After 3 hours she was finally seen by the ER doctor and he recommended discharge. When I asked about prednisone, he said he didn't feel comfortable prescribing it. When I asked if he could page the rheumatologist (we had no ability to contact her over the weekend) he declined to do so, suggesting we follow-up on Monday with our rheumatologist. I asked about the attending rheumatologist for St. Lukes, and he said there wasn't one since it was after hours. We made a huge mistake and accepted his judgement and didn't push back harder.

On Monday, October 15, Brittany went for her lab work at BCM. Her lab work came back later to show PR3 of 13 (negative), MPO of 30 (moderate positive), normal result for Lupus anticoagulent test, negative smith antibody, negative DNA (DS) antibody, negative cardiolipin antibodiesand positive ANCA (1:40 with P-ANCA titer). The rheumatologist saw her and sent her to the St. Lukes ER. After four hours of waiting there it finally clicked with me that I needed to be aggressive to get her adequate care. I got very assertive and angry with the ER doctor and she was given methylprednisone and a bed in the hospital.

With IV methylprednisone and later 60mg oral prednisone things improved. Her chest CT and cough improved. She got a kidney biopsy on October 18 and was released from the hospital October 19.

On October 24, an ANA test resulted positive with a 1:320 titer and mixed pattern. She also had normal Eosinophils, normal C3/C4. Kidney biopsy results supported ANCA vasculitis. At this point Dr. Bhairavarasu diagnosed her with ANCA-vasculitis which she believes is GPA.

Brittany started Rituximab infusions November 5, 2017 and finished her last one on November 26. These followed the RAVE protocol. Since then the lab work has shown a lot of improvement. As of early December her ANCA, MPO, and PR3 levels are normal. Her lung CT shows significant improvement, and protein and blood cell count in urine is decreased (close to 0).

Re: Origin Story

With proper treatment, it is possible to lead a normal life. Getting the treatment “just right” may require some tweaking.

The only advice I have at the moment is to taper off prednisone slowly. A too rapid taper may cause symptoms to return. It took me nine months to taper from 5 mg/day to zero, but I’ve been off the stuff since June 2018.

Good luck and keep us posted about progress.

Pete
dx 1/11

"Every day is a good day. Some are better than others." - unknown

"Take your meds as directed and live your life as fully as you can." - Michael Chacey, MD

Re: Origin Story

Well, I must say you've truly done your homework. Best of luck with your journey.

If I could pile on to Pete's advice, I will add the following:

--Get Brittany into an eye doctor (preferably an ophthalmologist, but you can start with an optometrist) and have her ocular pressure checked. Prednisone can cause a lot of damage to the eyes, but there are drops on the market that can at least hold the drug induced glaucoma at bay). I had very high pressures (greater than 40) that I've gotten down to the low teens (normal) with the right drops.

--There are going to be days were Brittany feels 100% and wants to take on more than she should. Make sure she paces herself and gets her rest. She may feel good that day, but going to hard may put her down for multiple days afterwards.

--Get Home Urine Test strips to check for Protein/Blood. These are the ones I use (I don't trust the off brands on amazon that have the potential to be cheap knockoffs)

Re: Origin Story

Hi Alex and Brittany,
Brittany, I am so sorry to hear you have this disease, but I am also happy to hear you have a partner like Alex who is sharing this journey with you. It is a frustrating one, and such a variety of symptoms occur, that it is hard to get a handle on it.
I will reiterate what Pete said. You will get to a point in tapering prednisone where it becomes difficult, and sometimes a flare may start. My number seems to be 10 mg. I made it to 9, dropping 1/4 increments every few weeks. I am currently at 8 3/4. In my case prednisone is a necessary evil. It has caused me to have sugar diabetes.
Also, what Richard said about pacing yourself is so true. After my first series of Retuxin infusions, I felt great. Returned to life as normal and then some. Within a month I was flat on my back, several antibiotics and an increased dosage of prednisone. This is a very deceptive disease, the numbers may look good, then whamo. You will find a new normal that works for you. My doctors initially told me that I would be on a regiment that works for me. It took almost two years but I now know what it takes to stay steady.
You being in Huston is great. You have access to a lot of medical facilities and good doctors.
I am in Charlotte, and am happy with my care. My ENT, who is a surgeon, diagnosed me immediately. My Rheumatologist suspects I have had this for years. I was diagnosed with asthma, severe sinus trouble, multiple cases of pneumonia, and many unnecessary sinus surgeries.
What you went through in the emergency room is inexcusable. We have that happen here too. When my husband had to go to the ER, I called the ambulance and he got immediate attention.
I wish I knew more about health insurance. I am on Medicare. You have a real conundrum there, since it is already a pre-existing condition.
One more thing, don’t be surprised about mood changes. Prednisone does that. If you are sleepless or tense, your Rheumatologist should be able to prescribe something. Many people on this site have felt the need for a professional to talk to, if it gets to that point.
I have a great husband, and two wonderful dogs. Dogs rock.
If Wegeners bothers your sinus or teeth I will be happy to share. When GPS hit my mouth I had a great deal of dental work done. I cried then, but now I flash my ‘Queen of the Implant’ smile.
Be well,
Masha

Re: Origin Story

With proper treatment, it is possible to lead a normal life. Getting the treatment “just right” may require some tweaking.

The only advice I have at the moment is to taper off prednisone slowly. A too rapid taper may cause symptoms to return. It took me nine months to taper from 5 mg/day to zero, but I’ve been off the stuff since June 2018.

Good luck and keep us posted about progress.

Thank you for welcoming us and congratulations on being prednisone free since June!

Originally Posted by richard052018

Well, I must say you've truly done your homework. Best of luck with your journey.

If I could pile on to Pete's advice, I will add the following:

--Get Brittany into an eye doctor (preferably an ophthalmologist, but you can start with an optometrist) and have her ocular pressure checked. Prednisone can cause a lot of damage to the eyes, but there are drops on the market that can at least hold the drug induced glaucoma at bay). I had very high pressures (greater than 40) that I've gotten down to the low teens (normal) with the right drops.

--There are going to be days were Brittany feels 100% and wants to take on more than she should. Make sure she paces herself and gets her rest. She may feel good that day, but going to hard may put her down for multiple days afterwards.

--Get Home Urine Test strips to check for Protein/Blood. These are the ones I use (I don't trust the off brands on amazon that have the potential to be cheap knockoffs)

--You have to trust your doctor 100%. If not, find another. You're in a big city with lots of options.

Again, Best of Luck!!

Thank you for the advice! We will reach out to an eye doctor. I also ordered the test strips. How frequently do you use them? Do you establish a baseline and then any deviation is an indicator of possible inflammation?

As far as rheumatologists go, does anyone have advice on how to select one? We stumbled into this doctor based on appointment availability. I would definitely prefer a second opinion.

Originally Posted by Masha

Hi Alex and Brittany,
Brittany, I am so sorry to hear you have this disease, but I am also happy to hear you have a partner like Alex who is sharing this journey with you. It is a frustrating one, and such a variety of symptoms occur, that it is hard to get a handle on it.
I will reiterate what Pete said. You will get to a point in tapering prednisone where it becomes difficult, and sometimes a flare may start. My number seems to be 10 mg. I made it to 9, dropping 1/4 increments every few weeks. I am currently at 8 3/4. In my case prednisone is a necessary evil. It has caused me to have sugar diabetes.
Also, what Richard said about pacing yourself is so true. After my first series of Retuxin infusions, I felt great. Returned to life as normal and then some. Within a month I was flat on my back, several antibiotics and an increased dosage of prednisone. This is a very deceptive disease, the numbers may look good, then whamo. You will find a new normal that works for you. My doctors initially told me that I would be on a regiment that works for me. It took almost two years but I now know what it takes to stay steady.
You being in Huston is great. You have access to a lot of medical facilities and good doctors.
I am in Charlotte, and am happy with my care. My ENT, who is a surgeon, diagnosed me immediately. My Rheumatologist suspects I have had this for years. I was diagnosed with asthma, severe sinus trouble, multiple cases of pneumonia, and many unnecessary sinus surgeries.
What you went through in the emergency room is inexcusable. We have that happen here too. When my husband had to go to the ER, I called the ambulance and he got immediate attention.
I wish I knew more about health insurance. I am on Medicare. You have a real conundrum there, since it is already a pre-existing condition.
One more thing, don’t be surprised about mood changes. Prednisone does that. If you are sleepless or tense, your Rheumatologist should be able to prescribe something. Many people on this site have felt the need for a professional to talk to, if it gets to that point.
I have a great husband, and two wonderful dogs. Dogs rock.
If Wegeners bothers your sinus or teeth I will be happy to share. When GPS hit my mouth I had a great deal of dental work done. I cried then, but now I flash my ‘Queen of the Implant’ smile.
Be well,
Masha

It's crazy to hear about all the symptoms you had leading up to your diagnosis. This is such a mysterious illness in how it affects people so differently. We love our dog too! Her name is Oatmeal.

Re: Origin Story

As for the Urine strips, the Cleveland Clinic recommends testing once a week. I test a little more than that, but this disease has turned me a little neurotic (wife is reaching her limits with me). Right after diagnosis, I had a patch of time where my strips indicated trace blood, but once I got my blood pressure (thanks pred) under control, I haven't had much in terms of blood/protein showing up on the strips (nor at the doctor). Hasn't stopped the creatinine from rising though (I'm frustrated to say the least).

Most people recommend having a local rheumatologist, plus going to one of the doctors recommended by the Vasculitis Foundation for additional guidance/2nd opinion. Hopkins, Mayo, Cleveland Clinic are near the top, as expected.